Patient representatives from the United Kingdom, Germany, and the United States have advocated for a change in the name of Primary Biliary Cirrhosis (PBC) to remove the term "cirrhosis," which is often inaccurate histologically, as well as having led to stigmatization of some of these patients. The term cirrhosis is often associated with other causes of liver diseases with particular social implications and as such patients with PBC are sometimes discriminated against in applications for insurance and jobs because of the misunderstanding of their condition this may result in individuals not being able to purchase a home or obtain health or life insurance.
As part of the process, patient representative groups met with experts during 2014 including those who gathered at the EASL Monothematic Conference on Primary Biliary Cirrhosis in Milan in May 2014. A group of interested experts in the disease had also had ongoing discussions and were supportive of the patient driven recommendation for a change in the name. A variety of options were considered through extensive email chains and an online survey put forth from the PBC Foundation in the UK and posted via the US PBCers organization website and via Facebook. It was recognized that maintaining the acronym of PBC was highly desirable and that there were no perfect terms for this condition. The two leading candidates were Primary Biliary Cholangitis and Primary Biliary Cholangiopathy with the term Primary Biliary Cholestasis also being discussed.
Additionally, there were polls from experts from Europe, the United States, and Japan, which was comprised of more than 50 individuals. Among this group there was a strong consensus, with 95 percent moving for changing the name. A similar large percentage, around 85 percent, recommended keeping the acronym. A clear preference for Primary Biliary Cholangitis, compared to Primary Biliary Cholangiopathy, by a margin of greater than 3:1, emerged from these discussions. The leadership of the other liver society is aware of the progress that has taken place in the name change discussion and has been supportive of the change in name to Primary Biliary Cholangitis.
Similarly, when patients were polled within their patient organizations, again there was an almost universal desire to see the term "cirrhosis" removed from the name. Like the experts, Primary Biliary Cholangitis was favored over Primary Biliary Cholangiopathy or Cholestasis due to its simplicity and pathological correctness, without other strong candidate terms emerging in the discussion. Because of this, there has been a move initiated by patients, supported by experts in the field to promote a change in name from Primary Biliary Cirrhosis to Primary Biliary Cholangitis. The acronym of PBC will remain.
One of the issues that continue to confound patients and some providers is the distinction between Primary Biliary Cirrhosis (now Cholangitis) and Primary Sclerosing Cholangitis. The two diseases have shared two out of three letters in their acronym, and now will be sharing two out of three words in their fully written names. This has the potential to add to the confusion and it will take a concerted educational effort by professional societies, key opinion leaders, as well as representative lay organizations to help ensure that the appropriate distinctions between the two conditions are made. Oftentimes the two conditions are considered in the differential of diagnosis with patients presenting cholestatic liver enzymes and many times drug therapies tested in one condition are tested in the other. There is a potential confusion between the two conditions and because the diseases have different courses, sets of complications, and at least for Primary Biliary Cholangitis, a specific treatment, the distinction remains an important one.Input was sought from PSC Partners Seeking a Cure and PSC Support UK who both point out that while the names are becoming more similar, the diseases remain distinct and require different clinical approaches with the need for increased funding and awareness to obviate any confusion or incorrect diagnosis. This naming similarity will create an increased need for both clinical and patient education to avoid future confusion. We in the liver community need to ensure that the change in name of PBC does not add to the misidentification or mismanagement of patients.